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Background: Anetoderma, characterized clinically by macular depressions or outpouchings of skin, is associated with loss of dermal elastic tissue as noted on histopathologic findings. We report on 9 extremely premature infants who developed patches of anetoderma during their course in the neonatal intensive care unit., Observations: All 9 patients were born between the ages of 24 and 29 weeks of gestation and had numerous complications associated with prematurity. Eight of the 9 infants were noted to have developed anetoderma on the trunk and proximal extremities while in the neonatal intensive care unit. The locations of the lesions on the skin were not explained by previous trauma, although many areas corresponded with placement of monitoring leads or with adhesive for a monitoring device. Reduction or absence of elastic tissue supported the diagnosis of anetoderma in 4 of 5 biopsy specimens., Conclusion: We report a previously unrecognized type of anetoderma associated with extreme prematurity. The exact cause is uncertain, although reactions to cutaneous monitoring leads or adhesives is suspected.

Background: Fibroblastic rheumatism was first described in the French literature in 1980. Since that time, 11 other patients with this disorder have been identified in the literature, mostly from France. This is a unique syndrome characterized by the sudden onset of symmetric polyarthritis and cutaneous nodules ranging from 5 to 20 mm in diameter, with predilection for the upper and lower extremities. While the cutaneous findings resolve spontaneously after several months, permanent joint sequelae are common., Observations: We present the first two patients with fibroblastic rheumatism reported in the United States. The clinical features and histologic findings were identical to those of patients described in the European literature., Conclusions: Fibroblastic rheumatism is a relatively rare syndrome characterized by the association of multiple cutaneous nodules with polyarthritis. The clustering of cases in France may, in part, be attributable to increased recognition by clinicians owing to prevalence of reports in the European literature. Perhaps, as clinicians in the United States become familiar with this disorder, further cases will be diagnosed, and the pathogenesis of the disorder will be elucidated.

Background: Elastosis perforans serpiginosa (EPS) is an uncommon skin disease characterized by transepidermal elimination of abnormal elastic fibers. The disease is frequently associated with congenital connective tissue disorders or Down's syndrome. The pathogenesis of EPS is still unclear. There are a few reports in the literature about a familial occurrence of EPS in which different modes of inheritance are suggested. To support the hypothesis of a congenital origin of the disease, we have studied another family with EPS., Observations: In this study, we describe a family in which two sisters and a brother were affected by EPS. The father and three paternal uncles were most probably affected by the same disease. There were no signs of other congenital connective tissue disease in the family members., Conclusion: An autosomal dominant mode of inheritance with variable expression of EPS is suggested.

Background and Design: Primary anetoderma is a rare cutaneous elastolytic disorder, the etiopathogenesis of which has not yet been established. Six patients with primary anetoderma were studied in an attempt to assess the role of the immunologic system in the elastolytic process. The investigation included the medical history, physical examination, routine blood tests, specific tests for collagen diseases, prothrombin time, activated partial thromboplastin time, thyroxine, indirect immunofluorescence test, and skin biopsies for histopathologic study and direct immunofluorescence., Results: Two of the patients presented with autoimmune disorders: the first had Graves' disease, lupus anticoagulant, and autoimmune hemolysis, and the second had systemic scleroderma. There were positive direct immunofluorescence findings in most of the patients. Furthermore, all of them were found to have serologic immunologic abnormalities, of which the most common was a positive antinuclear factor., Conclusions: These findings indicate that there is an immunologic involvement in primary anetoderma.

Background: Middermal elastolysis is a clinically and histologically distinct entity. This idiopathic loss of dermal elastic fibers has mostly been reported in younger adults. To our knowledge, the present case, which has been followed up for 4 years, is the first to occur in an elderly man., Observations: Two years after the onset of progressive wrinkling of the upper aspect of the thorax, the patient underwent a biopsy. Histologic examination of the specimens confirmed previous findings of middermal elastolysis. Examination of extracutaneous elastic tissue showed normal findings. Electron microscopy demonstrated elastic fibers embraced by macrophages, which is suggestive of elastic fiber phagocytosis. For the following 4 years, the patient has remained in stable clinical condition., Conclusions: Middermal elastolysis is probably more common than has been assumed so far. It does not affect nondermal elastic tissue. After progressive loss of dermal elasticity in a circumscribed area, a benign course follows, with a stable condition over several years. Electron microscopic findings indicate that elastic fiber phagocytosis is operative in the disappearance of the middermal elastic tissue.

We studied an 86-year-old Japanese man with linear focal elastosis. The lesions were asymptomatic yellow striae in the lumbar region, histologically composed of massive, well-demarcated basophilic fibers that stained positively with elastic tissue stains. Electron microscopy revealed fine, reticular or granular electron-dense materials, and elastic fiber microfibril-like materials in the matrix, in addition to numerous mature and immature elastic fibers. These findings suggest that active elastogenesis was occurring in the lesions. The four cases reported so far have the three common features of age, sex, and lesion location.

A patient had acquired perforating dermatosis and suffered from renal disease, diabetes mellitus, and lupus vulgaris. Histologic and immunohistochemical studies revealed that the bulk of the coarse granular basophilic material being extruded by transepidermal elimination was of nuclear origin obviously derived from polymorphonuclear leukocytes that were particularly abundant in an early, nonperforated lesion. At the lower boundary of the material being eliminated transepidermally, leukocytes were seen to accumulate, to undergo pyknosis and karyorrhexis, and to transform into nuclear debris. As a minor component, the material contained collagen fibers with altered staining qualities and, in an early lesion, elastic fibers. We speculate that accumulation, disintegration, and enzyme release from polymorphonuclear leukocytes may represent an important, hitherto disregarded driving force in transepidermal elimination. Lysosomal enzymes may later be responsible for the alteration of staining properties in collagen fibers, the degradation of elastic fibers, and for opening up the transepidermal route by impairing intercellular keratinocyte cohesion.

One hundred thirty-three patients were examined for the presence of a cutaneous eruption consisting of small, asymptomatic, hypopigmented, follicular papules on the upper part of the trunk. The papules were present in 57% of patients who had a history of acne vulgaris and in 9% of patients who denied ever having had acne. Biopsy specimens from five patients revealed circumscribed, perifollicular or parafollicular lesions in which both elastic and collagen fibers were attenuated in comparison with those in normal adjacent dermis. These changes are consistent with scar. We conclude that the papules are a scarring process secondary to acne vulgaris and propose calling the lesions papular acne scars. They appear closely related to or identical with changes previously termed perifollicular elastolysis, postacne anetoderma-like scars, and papular elastorrhexis.

Elastolytic cutaneous lesions were the initial manifestation of amyloidosis in two patients with multiple myeloma. The skin of the fingertip was noticeably soft, loose, and redundant. The skin of the fingers was depressed with pressure and remained depressed for an abnormally long period of time. Amyloid deposition in the dermis was demonstrated in both cases. In addition, fragmentation and a decrease of elastic fibers in the dermis were observed. Possible explanations for the pathogenesis of such skin lesions were also discussed.

Histologic paraffin sections of pseudoxanthoma elasticum (PXE)-involved skin of forearm and axilla were used for histochemistry and immunohistochemical and analytical electron microscopy to study the progressive mineralization in the dermis of patients with PXE. The von Kossa technique identified mineral deposits throughout the reticular PXE dermis. X-ray analysis revealed patterns of calcium and phosphorus deposition in the von Kossa-positive areas, and the immunohistochemical staining using monoclonal antibodies identified increased chondroitin-6-sulfate in these areas when compared with normal skin. Scanning transmission electron microscopy observation combined with X-ray dot mapping show calcium and phosphorus to be codistributed within the mineralized area. This study confirms by new methods the increase in chondroitin-6-sulfate, alterations in elastin and collagen, and a high calcium and phosphorus elemental distribution matching the mineralized area in the PXE dermis.

A progressive staining technique is described that will permit demonstration of elastic structures and amyloid deposits in the same section. It is satisfactory for animal as well as human tissues, and for both pericollagen and perirecticulin types of amyloidosis.

We describe a 33-year-old woman with idiopathic loss of middermal elastic tissue, resulting in widespread fine wrinkling of the skin. This represents an example of the rarely reported entity middermal elastolysis. The literature is reviewed.

Skin biopsy specimens from 21 patients with Ehlers-Danlos syndrome (EDS) were compared with controls. With two exceptions, the appearance of the dermal collagen and elastic tissue as seen in the two groups was indistinguishable. One example of type 4 EDS contained a dermis composed of fibers that resembled actinically damaged elastic tissue. The single example of type 6 EDS contained particularly thin collagen fibers. The dermal thickness of specimens of EDS was similar to that of controls, although the abnormal-appearing specimen of type 4 EDS was also abnormally thin. Since the other two biopsy specimens of type 4 appeared to be within the range of normal, there may be heterogeneity in this form of EDS.

A case of follicular occlusion disease with notably increased and abnormal elastic tissue and transepidermal elimination of elastic fibers is reported. This combination of findings has traditionally been regarded as unique to elastosis perforans serpiginosa and, to our knowledge, has not previously been described in other follicular diseases. It is hypothesized that the histopathologic changes seen in this case might be a nonspecific secondary alteration in the dermis caused by long-standing chronic inflammation and/or repeated exposure of the dermis to follicular contents.

Patients with renal disease or diabetes mellitus often have an acquired perforating disease of the skin develop that is characterized by hyperkeratotic papules with transepidermal elimination of degenerated material, including collagen or elastic fibers. There is disagreement regarding the most appropriate name for this disease. The pathologic process has been identified by various authors as reactive perforating collagenosis, elastosis perforans serpiginosa, perforating folliculitis, or Kyrle's disease. We have seen four patients with renal disease and/or diabetes whose skin biopsy specimens demonstrated combined transepidermal elimination of both collagen and elastic fibers. This finding is not characteristically seen in any of the previously defined perforating diseases. Since the histologic findings vary greatly in different lesions from different patients with renal disease, we recommend referring to this process as "acquired perforating dermatosis." It is best not to create a new category of perforating disease or to say that a given patient has one of the other four diseases based on random sampling of only a few lesions.

We encountered a case of elastosis perforans serpiginosa in a patient with coexisting renal disease, the first such reported association of which we are aware. The perforating disorders tend to have atypical clinical features when associated with renal disease. The exact pathogenic mechanism by which renal disease predisposes to a perforating disease remains to be elucidated but is likely to be related to minor trauma, which occurs in renal disease.

A 56-year-old woman had pseudoxanthoma elasticum (PXE). Ultrastructural findings in the endocardium showed increased collagenogenesis and elastogenesis. The newly formed elastic fibers appeared morphologically normal, but showed a notable propensity to become calcified. The internal structure of the calcified areas was essentially similar to that of the adjacent uninvolved portions of the elastic fibers. Some of the heavily calcified fibers showed loss of internal structure and fragmentation. These findings support the concept that in PXE, calcification involves morphologically unaltered elastic fibers that subsequently may undergo secondary degenerative changes, such as loss of internal structure and fragmentation.

Ring-shaped inflammatory lesions sometimes develop in the abnormal "elastotic" connective tissues of skin damaged by sun and heat. The lesions, which commence as papules and nodules, enlarge very slowly and may persist for years. Microscopical sections show that there is an infiltrate composed mainly of foreign-body giant cells, the cells being engaged in digesting and absorbing the abnormal elastotic fibers. The disorder, which occurs on several continents, should probably be regarded as a phenomenon of repair within damaged connective tissue. The name actinic granuloma indicates its external or environmental origin and distinguishes it from other granulomas with which it is constantly being confused. Actinic granuloma and granuloma annulare appear to be related. In granuloma annulare, a productive and resorptive process also occurs, but its nature remains obscure. Actinic granuloma may be misdiagnosed as "atypical necrobiosis lipoidica" or as a sarcoidosis. The subject of actinic damage to blood vessels deserves study.

We describe a patient with a 3-cm sessile tumor of the heel. Despite its large size and unusual location, it had the histologic characteristics of a fibrokeratoma. The essential microscopic feature of fibrokeratoma is its core of collagenous fibers with both interwoven and parallel arrangements. Epidermal hyperplasia and hyperkeratosis are often present, but probably represent secondary changes. Fibrokeratomas, therefore, have some of the clinical and pathologic features of collagenomas.

We reviewed and reclassified the histology in 207 cases of granuloma annulare from our files. The most common pattern found was mononuclear infiltrative type (72%) in contrast to the palisading group (25%) and the epithelioid nodules (3%). Indeed, and entire spectrum from mononuclear infiltrates to the epithelioid sarcoidal types is seen. The electron microscopic findings include histiocytes with membrane activity, well-developed rough endoplasmic reticulum, large mitochondria, and numerous lysosomes. Pathcy damage of collagen and elastic fibers with the presence of vesicles, lysosomes, and cytoplasmic debris are demonstrated. The histochemical and ultrastructural studies localized acid phosphatase in the primary lysosomes and the routh endoplasmic membrane. The presence of fibrin deposition confirmed our previous immunofluorescence results.

A patient with dermatofibrosis lenticularis disseminata (Buschke-Ollendorff syndrome) was found to have a distinctive abnormality of the cutaneous elastic tissue. This abnormality, studied by histochemical and ultrastructural techniques, was characterized by the presence of hyalinized, thick fibers that entrapped normal bundles of collagen. Histochemically, these fibers stained like elastin but showed certain tinctorial characteristics of pre-elastin. Ultrastructurally, this elastin-like substance was composed of large clumps of electron-dense material with a fine fibrillar coating. The changes were sufficiently distinctive to be diagnosed by light microscopy.

Elastic fibers account for 2% of dermal volume and are responsible for normal skin resiliency. We investigated a disorder of the dermal elastic component as the mechanism for the decreased elasticity of skin in myxedema. Skin biopsy specimens were obtained from patients with thyroid diseases and normal subjects matched for age, sex, and biopsy location. Elastic fiber concentration, determined by computerized morphometric analysis of Verhoeff-van Gieson-stained sections, was significantly lower than normal in hypothyroid and pretibial myxedema. The decreased elastin concentration was not a consequence of the glycosaminoglycan infiltration. Ultrastructural studies of myxedematous skin showed wide variability of elastic fiber diameter and decreased microfibrils. Myxedema (hypothyroid and pretibial) is consistently associated with quantitative and qualitative defects of dermal elastic fibers.

Two cases of actinic granuloma are described with emphasis on distinctive clinical and histopathologic features, including immunoperoxidase staining for lysozyme and immunophenotyping of mononuclear leukocytes. Actinic granuloma presents in chronically sun-damaged skin as normally colored to erythematous papules that coalesce to form centrifugally enlarging annular patterns. By light microscopy, a granulomatous infiltrate of giant cells and histiocytes is seen to be intimately related to the presence of elastotic fibers in the upper dermis. Selective localization of lysozyme in the giant cells of the granuloma is apparent by a tertiary antibody immunoperoxidase technique. Determination of mononuclear leukocyte subsets with monoclonal antibodies reveals a predominance of helper T cells in the lymphocytic infiltrate associated with the granuloma. It is postulated that actinic granuloma represents a cell-mediated immune response to weakly antigenic determinants on actinically altered elastotic fibers.